SLE and Avascular Necrosis Creates an Educated Consumer

A young lady sitting next to me on a plane held out a bottle of water and asked me to open it. We were traveling from Philadelphia to Arizona - she for her sister's wedding and I on a business trip. As I twisted the cap, she said she couldn't unscrew it due to a medical condition that affected the strength in her hands. She said she had systemic lupus erythematosis (SLE) and had been treated with steroids. I was fascinated by her story.

She told me three years earlier she was diagnosed with cancer. Her mother told the physician "no way does my daughter have cancer." The mother asked for a second opinion- who correctly diagnosed lupus and treated her daughter appropriately with steroids. After two years, she developed avascular necrosis (AVN), or osteonecrosis of both knees; it is death of bone tissue due to a lack of blood supply. Without blood, the bone tissue dies and the bone collapses. If avascular necrosis involves the bones of a joint, it often causes destruction of the articular surfaces. AVN commonly affects the ends (epiphysis) of long bones such as the femur (the bone extending from the knee joint to the hip joint). The most common involvement is the distal femur or the thigh bone portion of the knee. It is not uncommon for the tibia or shinbone also to be involved, but it is unusual for the patella (kneecap) to be involved.

A risk factor for AVN is excessive steroid use- it isn't clear how these anti-inflammatory medications, such as prednisone, cause avascular necrosis. AVN is also a complication of lupus. Since early in the disease x-ray images usually appear normal, bone scintigraphy and MRI are the diagnostic modalities of choice.

Her physician told her she needed a total knee replacement (TKR); she was 20 years old. She is very smart and teaches underprivileged students in Philadelphia, so she searched the internet for other options and found an orthopedic surgeon in Canada. He told her she was "too young" to have a TKR; instead, he recommended a partial knee replacement, which would be much less debilitating for her recovery would be much faster.

The message of this story is that SLE can be tricky to diagnose and it pays for a patient to be "an educated consumer" and to seek a second opinion, if there is any doubt, especially when it comes to surgical procedures.